BCAT2 and Glucose intolerance: In murine models, impaired BCAA catabolism, resulting from deficient mitochondrial enzymes such as branched-chain keto acid dehydrogenase e1 α subunit (BCKDHA) or branched-chain amino acid transaminase 2 (BCAT2), leads to elevated plasma BCAAs, reduced insulin sensitivity, glucose intolerance, and weight gain [90,113,114].